Disorders of airways

Question 1

Cystic fibrosis- Eti

Answer 1

• Most common fatal hereditary disorder.
• 1 in 3200 whites
• 1000 mutations in CFTR gene
• S. aureus & P. aeruginosa

Question 2

Cystic fibrosis- Sx

Answer 2

• Chronic/ recurrent cough, sputum, dyspnea, wheezing
• Recurrent infections/ colonization
• Bronchiectasis & scarring x-ray
• Pancreatic insufficiency, hepatic disease, nutritional deficiency (low mass index)
• Rhinosiunsitis, steatorrhea, diarrhea, abd pain
• Absence of vas deferens

Question 3

Cystic fibrosis- Dx

Answer 3

• Sweat chloride concentration
• genetic testing
• PFT: obstructive/ restrictive pattern
• Arterial blood gas = hypoxia

Question 4

Cystic fibrosis- Tx

Answer 4

• Bronchodilators, abx, inhaled saline, inhaled recombinant DNas- thins sputum

Question 5

Cystic fibrosis- Prog/ Prev

Answer 5

• Pneumovax, flu, genetic screening

- Median survival >35 yrs

Question 6

Bronchiectasis- Eti

Answer 6

• Congenital/ acquired disorder of large bronchi- abn dilation of bronchial walls
• Inflammation, infection or cystic fibrosis (50%)

Question 7

Bronchiectasis- Sx

Answer 7

• Cough + copious purulent sputum
• Recurrent pnemonia
• Wt loss & anemia
• Pleuritic chest pain
• Clubbing

Question 8

Bronchiectasis- Common organisms

Answer 8

• H. influenza most common

- P. aeruginosa, S. pnemoniae, S. aureus

Question 9

Bronchiectasis- Dx

Answer 9

• CT- dilated, thickened bronchi

- CXR- tram tracks, dilated, thickened bronchi, scattered opacities, atelectasis, consolidation

Question 10

Bronchiectasis- Tx

Answer 10

• Acute- Emperilcal abx

- Long term macrolide or alternating amoxicillin